Syringomyelia

What Is Syringomyelia?

Syringomyelia is a rare disorder in which a cyst forms within your spinal cord. As the cyst, which is called a syrinx, expands and lengthens over time, it compresses and damages part of your spinal cord from its center outward.

Damage to the spinal cord caused by a syrinx can cause symptoms like progressive pain, stiffness, and weakness in the back, shoulders, arms, and legs. Individuals with the disorder might lose the ability to feel cold and pain normally. Some people with this disorder will not have any symptoms and will not need treatment. For others, syringomyelia will cause symptoms and complications that worsen as the syrinx expands.

Treatment aims to relieve the pressure on your spinal cord. The treatment your doctor suggests for you will depend on the cause of your syringomyelia. Follow-up care after surgery is important because syringomyelia can reoccur.

What Causes Syringomyelia?

Most cases of syringomyelia are caused by a malformation of the brain known as a Chiari type 1 malformation (CM1). A CM1 occurs where the brain joins the spinal cord; in this malformation, the cerebellum (brainstem) lies lower than normal. Often the cerebellar tonsils (the base of each lobe of the cerebellum) protrude from the skull and into the spinal canal.

Syringomyelia can develop as a complication of trauma, meningitis, hemorrhage, a tumor, or arachoiditis. Arachoiditis is a progressive inflammatory disorder that affects the arachoid or middle membrane that surrounds the brain and spinal cord. A primary arachoid cyst is present at birth, but it may take years for symptoms to appear.

What Are the Symptoms of Syringomyelia?

Symptoms of the disorder are caused by the pressure the syrinx puts on the spinal cord and the damage that follows. The symptoms may include:

progressive weakness and pain in back, shoulders, arms, or legs

inability to feel hot or cold normally

loss of pain sensations

difficulty in walking

bowel and bladder function problems

facial pain and numbness

scoliosis (curvature of the spine)

If you have any of these symptoms, you should visit your doctor. If you have had a spinal injury, it is important to watch for these symptoms. It may take months or even years after your injury for syringomyelia to develop.

How Is Syringomyelia Diagnosed?

If you doctor suspects you have syringomyelia, he may refer you to a neurologist. To diagnose your condition, your neurologist will first take your complete medical history. He or she will also give you a complete physical examination. Be prepared to tell your neurologist about your symptoms and how long you have had them.

If your neurologist thinks you may have syringomyelia, he or she will order a magnetic resonance imaging (MRI) test to look for a syrinx in your spinal cord. A MRI is the most reliable diagnostic tool for syringomyelia. In the past, a computer tomography (CT) scan or myelogram, which uses X-ray images with a constrast dye, were used to diagnose the disorder. Since the invention of the MRI, it is considered the gold standard for diagnosing syringomyelia.

How Is Syringomyelia Treated?

Treatment depends on the progression of the disorder and whether you are experiencing symptoms that disrupt your life. If you have no symptoms or mild symptoms, you will not need treatment; your neurologist will monitor the progression of the disorder.

If your symptoms are negatively affecting your life, your neurologist will recommend surgery. The goal of surgery is to relieve the pressure on your spinal cord. The type of surgery will depend on the cause of your syringomyelia.

If you have a CM1 malformation, your surgeon may suggest surgery to expand the base of your skull and the covering of your brain. This will take pressure off your spinal cord and your brain. The normal flow of cerebrospinal fluid should be restored. For most people, this surgery resolves their syringomyelia.

In some cases, your surgeon will use a shunt to drain the syrinx. The shunt, a small, flexible tube, is placed in the syrinx, and excess fluid is drained. Sometimes the surgeon is able to completely drain the syrinx during surgery. If not, the shunt remains in place after your surgery.

If you have a tumor or bony growth that is causing syringomyelia, removal of the growth may resolve the syringomyelia.

After surgery you may be prescribed a course of antibiotics to prevent complications from infection. Physical therapy can help strengthen muscles in limbs that suffered progressive weakness.

What Is the Outlook for People With Syringomyelia?

The outlook of those who undergo treatment and have a successful surgery depends on each case. Damage to the spinal cord may be severe enough to cause permanent neurological issues. Some people may struggle with walking or have permanent weakness in their limbs. The hope is these conditions will slowly disappear with physical therapy and time.

It is important to keep all follow-up appointments with your doctor. You will need periodic MRIs because syringomyelia can reoccur.

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